{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T07:02:49Z","timestamp":1789542169743,"version":"build-2803163510"},"reference-count":18,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T00:00:00Z","timestamp":1789430400000},"content-version":"vor","delay-in-days":257,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Case Reports in Pediatrics"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Identifying the cause of protein\u2010losing enteropathy (PLE) is crucial for appropriate treatment. Primary intestinal lymphangiectasia (PIL) is a cause of PLE, and low\u2010strict fat diet with medium\u2010chain triglyceride (MCT) supplementation may be insufficient. Sirolimus has shown efficacy in refractory PIL; however, its use in infants remains uncommon, and the optimal timing of initiation has not been established.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>\n                      A previously healthy 7\u2010month\u2010old Japanese male infant presented with convulsions, generalized edema, and diarrhea. Laboratory findings revealed severe hypoproteinemia (serum albumin: 1.3\u2009g\/dL), lymphopenia (absolute lymphocyte count: 822\/\u03bcL), and electrolyte abnormalities. Scintigraphy using\n                      <jats:sup>99m<\/jats:sup>\n                      Tc\u2010labeled human serum albumin showed tracer exudate in the small intestine, leading to the diagnosis of PLE. Upper endoscopy revealed snowflake\u2010like white spots in the duodenum, and pathological examination confirmed lymphatic dilation, leading to a PIL diagnosis. Despite treatment with a low\u2010strict fat diet with MCT supplementation and corticosteroids, hypoproteinemia persisted. Lymphatic scintigraphy and magnetic resonance lymphangiography confirmed ongoing protein leakage. Sirolimus was initiated at 0.5\u2009mg\/day on Day 23. Subsequently, serum albumin began to increase within 1\u20132\u2009weeks and ultimately reached 4.1\u2009g\/dL, and absolute lymphocyte count, 1917\/\u03bcL immediately before sirolimus initiation, increased to 3843\/\u03bcL. Lymphatic scintigraphy on Day 74 of sirolimus treatment showed complete resolution of protein leakage. Sirolimus was continued for 121\u2009days and discontinued thereafter. At 17\u2009months of follow\u2010up, no recurrence was observed, with sustained normalization of serum albumin to 4.0\u2009g\/dL and absolute lymphocyte count to 4739\/\u03bcL.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>This case suggests one of the earliest reported responses to sirolimus in the treatment of PIL, with improvement observed within a few weeks of initiation. Early introduction of sirolimus in patients with persistent protein leakage resistant to low\u2010strict fat diet with MCT supplementation may be associated with rapid improvement in PIL, though this hypothesis warrants further case accumulation.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1155\/crpe\/7717714","type":"journal-article","created":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T06:11:22Z","timestamp":1789539082000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Successful Early Therapy With Sirolimus for an Infant With Primary Intestinal 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